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Idiopathic Pulmonary Fibrosis

Jeremy Feldman, MD

  • What is Idiopathic Pulmonary Fibrosis?
    • Idiopathic Pulmonary Fibrosis Definition
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and Idiopathic Pulmonary Fibrosis
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy and Prognosis for Idiopathic Pulmonary Fibrosis
  • Diagnosing & Monitoring PF
    • Monitoring Progress of IPF
    • Idiopathic Pulmonary Fibrosis Diagnosis
    • Chest X-rays Used in IPF
    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
    • Complications of Idiopathic Pulmonary Fibrosis
  • PF Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
    • Pulmonary Rehab for IPF Patients
    • Lung Transplantation for Pulmonary Fibrosis
    • Oxygen Used to Treat IPF
      • Update on Oxygen Use
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation
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  • FAQ’s
    • Respiratory failure, NSIP, diet
    • Leg swelling, lung biopsy, side effects & pets
    • FAQ’s Enzyme Therapy, Progression of IPF & Oxygen
    • FAQ: Diarrhea, Fibrosis Reversal, IPF Symptoms
    • FAQ: Oxygen Flow, Anesthesia & Genetics
    • FAQ: Contagious Cough? Stem Cells, Familial IPF, Flying
    • FAQ: GERD, Fatigue & Environmental Exposure
    • FAQ: How to Travel to Your Doctor’s Office with Oxygen
    • FAQ: Lung Biopsy with Pulmonary Fibrosis
    • FAQ’s: Alcohol, Clinical Trials, Pulmonary Function Testing
    • FAQs: Emphysema, Exercise & Lung Biopsy

Rheumatoid Arthritis and Pulmonary Fibrosis

January 10, 2019 By Dr. Jeremy Feldman

rheumatoid arthritis and pulmonary fibrosisRheumatoid arthritis is an inflammatory disease of the joints.  It typically involves the hands, wrists and feet but can involve any joint.  What is less well known is that Rheumatoid Arthritis can involve the lungs in many ways.

  • Pulmonary Fibrosis:  scarring of the lungs
  • Bronchiolitis Obliterans:  an airway disease that behaves like severe asthma
  • Rheumatoid Nodules:  collections of inflammation in the lung tissue
  • Follicular Bronchiolitis:  airway inflammation with tiny nodules of lymph tissue
  • Pulmonary Hypertension:  high pressure in the blood vessels of the lungs

Symptoms of Pulmonary Fibrosis with Rheumatoid Arthritis

When Rheumatoid Arthritis leads to Pulmonary Fibrosis, patients often have severe symptoms of shortness of breath.  Over the years there have been few good quality studies of medications to treat this process.  Generally speaking, most medications that treat joint inflammation have been entirely ineffective at treating the scarring in the lungs.  Medications such as Humira, Enbrel and others just don’t work to stop the fibrosis.  We often try medications such as mycophenolate (Cell Cept) but results are usually disappointing as well.

A recent study of patients with Rheumatoid Arthritis (RA) and Pulmonary Fibrosis is shedding some light on why this is such a difficult problem to treat. Researchers have discovered that patients with RA and Pulmonary Fibrosis have mutations in a gene called MUC5B.

Idiopathic Pulmonary Fibrosis?

Interestingly, mutations in this gene have also been found in some patients with Idiopathic Pulmonary Fibrosis.  This further suggests that perhaps RA associated Pulmonary Fibrosis should be thought of more as IPF in a patient with RA.  Although there have not been any studies with OFEV or Esbriet we remain very interested in this as a better treatment for this disease.

Filed Under: IPF Diagnosis

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FAQ’s: Enzyme Therapy, Hereditary IPF, Progression of IPF & Oxygen

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Idiopathic Pulmonary Fibrosis Treatment Options

What is IPF?

  • What is Idiopathic Pulmonary Fibrosis?
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and IPF
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy & Prognosis for Pulmonary Fibrosis

Diagnosing IPF

  • Idiopathic Pulmonary Fibrosis Diagnosis
    • Chest X-rays Used in IPF
    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
    • Complications of Idiopathic Pulmonary Fibrosis

Treating IPF

  • Pulmonary Fibrosis Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
    • Pulmonary Rehab for IPF Patients
    • Lung Transplantation for Pulmonary Fibrosis
    • Oxygen Used to Treat IPF
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation

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